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词条 Necrolytic migratory erythema
释义

  1. Signs and symptoms

     Clinical features  Associated conditions 

  2. Cause

  3. Diagnosis

     Histology 

  4. Management

  5. See also

  6. References

  7. External links

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Necrolytic migratory erythema (NME) is a red, blistering rash that spreads across the skin. It particularly affects the skin around the mouth and distal extremities; but may also be found on the lower abdomen, buttocks, perineum, and groin. It is strongly associated with glucagonoma, a glucagon-producing tumor of the pancreas, but is also seen in a number of other conditions including liver disease and intestinal malabsorption.

Signs and symptoms

Clinical features

NME features a characteristic skin eruption of red patches with irregular borders, intact and ruptured vesicles, and crust formation.[1] It commonly affects the limbs and skin surrounding the lips, although less commonly the abdomen, perineum, thighs, buttocks, and groin may be affected.[1] Frequently these areas may be left dry or fissured as a result.[1] All stages of lesion development may be observed synchronously.[4] The initial eruption may be exacerbated by pressure or trauma to the affected areas.[1]

Associated conditions

William Becker first described an association between NME and glucagonoma in 1942[2][3] and since then, NME has been described in as many as 70% of individuals with a glucagonoma.[4] NME is considered part of the glucagonoma syndrome,[5] which is associated with hyperglucagonemia, diabetes mellitus, and hypoaminoacidemia.[2]

When NME is identified in the absence of a glucagonoma, it may be considered "pseudoglucagonoma syndrome".[6] Less common than NME with glucagonoma, pseudoglucagonoma syndrome may occur in a number of systemic disorders:[7]

  • Celiac disease
  • Ulcerative colitis
  • Crohn's disease
  • Hepatic cirrhosis
  • Hepatocellular carcinoma
  • Lung cancer, including small cell lung cancer
  • Tumors that secrete insulin- or insulin-like growth factor 2
  • Duodenal cancer

Cause

The cause of NME is unknown, although various mechanisms have been suggested. These include hyperglucagonemia, zinc deficiency, fatty acid deficiency, hypoaminoacidemia, and liver disease.[2] The pathogenesis is also unknown.

Diagnosis

Histology

The histopathologic features of NME are nonspecific[8] and include:[9]

  • epidermal necrosis
  • subcorneal pustules
  • confluent parakeratosis, epidermal hyperplasia, and marked papillary dermal hyperplasia in a psoriasiform pattern
  • angioplasia of papillary dermis
  • suppurative folliculitis

The vacuolated, pale, swollen epidermal cells and necrosis of the superficial epidermis are most characteristic.[2] Immunofluorescence is usually negative.[2]

Management

managing the original condition, glucagonoma, by octreotide or surgery.

See also

  • List of cutaneous conditions

References

1. ^{{cite journal |vauthors=Thiers BH, Sahn RE, Callen JP |title=Cutaneous manifestations of internal malignancy |journal=CA – A Cancer Journal for Clinicians |volume=59 |issue=2 |pages=73–98 |year=2009 |pmid=19258446 |doi=10.3322/caac.20005}}
2. ^{{cite journal |vauthors=Pujol RM, Wang CY, el-Azhary RA, Su WP, Gibson LE, Schroeter AL |title=Necrolytic migratory erythema: clinicopathologic study of 13 cases |journal=International Journal of Dermatology |volume=43 |issue=1 |pages=12–8 |date=January 2004 |pmid=14693015 |doi= 10.1111/j.1365-4632.2004.01844.x}}
3. ^{{cite journal |vauthors=Becker WS, Kahn D, Rothman S |title=Cutaneous manifestations of internal malignant tumors |journal=Archives of Dermatology and Syphilology |volume=45 |issue=6 |pages=1069–1080 |year=1942 |pmid= |doi= 10.1001/archderm.1942.01500120037004|url=}}
4. ^{{cite journal |vauthors=van Beek AP, de Haas ER, van Vloten WA, Lips CJ, Roijers JF, Canninga-van Dijk MR |title=The glucagonoma syndrome and necrolytic migratory erythema: a clinical review |journal=Eur. J. Endocrinol. |volume=151 |issue=5 |pages=531–7 |date=November 2004 |pmid=15538929 |doi= 10.1530/eje.0.1510531|url=http://eje-online.org/cgi/pmidlookup?view=long&pmid=15538929}}
5. ^{{cite book |author=Odom, Richard B. |author2=Davidsohn, Israel |author3=James, William D. |author4=Henry, John Bernard |author5=Berger, Timothy G. |author6=Clinical diagnosis by laboratory methods |author7=Dirk M. Elston |title=Andrews' diseases of the skin: clinical dermatology |publisher=Saunders Elsevier |location= |year=2006 |pages=143 |isbn=978-0-7216-2921-6 |oclc= |doi= |accessdate=}}
6. ^{{cite journal |vauthors=Marinkovich MP, Botella R, Datloff J, Sangueza OP |title=Necrolytic migratory erythema without glucagonoma in patients with liver disease |journal=Journal of the American Academy of Dermatology |volume=32 |issue=4 |pages=604–9 |date=April 1995 |pmid=7896950 |doi= 10.1016/0190-9622(95)90345-3|url=http://linkinghub.elsevier.com/retrieve/pii/0190-9622(95)90345-3}}
7. ^{{cite journal |vauthors=Mignogna MD, Fortuna G, Satriano AR |title=Small-cell lung cancer and necrolytic migratory erythema |journal=The New England Journal of Medicine |volume=359 |issue=25 |pages=2731–2 |date=December 2008 |pmid=19092164 |doi=10.1056/NEJMc0805992 |url=}}
8. ^{{cite journal |author=Wilkinson DS |title=Necrolytic migratory erythema with carcinoma of the pancreas |journal=Transactions of the St. John's Hospital Dermatological Society |volume=59 |issue=2 |pages=244–50 |year=1973 |pmid=4793623 |doi= |url=}}
9. ^{{cite journal |vauthors=Kheir SM, Omura EF, Grizzle WE, Herrera GA, Lee I |title=Histologic variation in the skin lesions of the glucagonoma syndrome |journal=The American Journal of Surgical Pathology |volume=10 |issue=7 |pages=445–53 |date=July 1986 |pmid=3014912 |doi= 10.1097/00000478-198607000-00001|url=}}

External links

{{Medical resources
| DiseasesDB = 8833
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| eMedicineSubj = derm
| eMedicineTopic = 168
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}}{{Urticaria and erythema}}{{Paraneoplastic syndromes}}

2 : Erythemas|Rare diseases

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